Greco Tommaso, Polichetti Chiara, Cannella Adriano, La Vergata Vincenzo, Maccauro Giulio, Perisano Carlo
Department of Ageing, Neurosciences, Head-Neck and Orthopedics Sciences, Orthopedics and Traumatology, Fondazione Policlinico Universitario Agostino Gemelli IRCSS Rome, Italy.
Orthopedics and Traumatology, Università Cattolica Del Sacro Cuore Rome, Italy.
Am J Blood Res. 2021 Jun 15;11(3):206-216. eCollection 2021.
Hemophilia is a bleeding disorder characterized by the deficiency of a coagulation factors. The hemarthrosis is the most common and earliest manifestation. Repeated hemarthrosis over time causes the development of hemophilic arthropathy. Among most involved joints, the ankle is the one where much uncertainty remains about the best course of action in managing the various degrees of hemophilia manifestations. These manifestations range from simple acute swelling and pain to devastating deformity. The purpose of our review is to draw a comprehensive picture of ankle hemophilic arthropathy epidemiology, pathophysiology, clinical symptoms and signs, radiological features and all the treatments available at present days. This review confirms that the first line of treatment considered should be the replacement therapy of the coagulation deficient factors that, preventing hemarthrosis, stops the development and progression of ankle's joint damage. The treatments proposed in literature for advanced stage of arthropathy are many and vary according to the severity of the case. They range from conservative ones such as physiotherapy, orthosis, intra-articular injections, laser therapy, external beam radiation therapy, radio-synovectomy and oral drug to invasive surgical treatment such as ankle arthrodesis and total ankle replacement. Whatever is the chosen treatment, according to the arthropathy severity we believe that it must be carried out in reference centers for foot and ankle surgery assisted by expert hematologists.
血友病是一种以凝血因子缺乏为特征的出血性疾病。关节积血是最常见且最早出现的表现。随着时间的推移，反复的关节积血会导致血友病性关节病的发展。在最常受累的关节中，踝关节对于处理不同程度血友病表现的最佳治疗方案仍存在诸多不确定性。这些表现从简单的急性肿胀和疼痛到严重的畸形不等。我们综述的目的是全面了解踝关节血友病性关节病的流行病学、病理生理学、临床症状和体征、放射学特征以及目前所有可用的治疗方法。本综述证实，首先应考虑的治疗方法是补充缺乏的凝血因子，通过预防关节积血来阻止踝关节损伤的发展和进展。文献中针对关节病晚期提出的治疗方法众多，且因病例严重程度而异。它们包括保守治疗，如物理治疗、矫形器、关节内注射、激光治疗、外照射放疗、放射性滑膜切除术和口服药物，以及侵入性手术治疗，如踝关节融合术和全踝关节置换术。无论选择何种治疗方法，根据关节病的严重程度，我们认为必须在足踝外科参考中心，在专业血液科医生的协助下进行。
